Literature DB >> 23223629

Nonequilibrium gating of CFTR on an equilibrium theme.

Kang-Yang Jih1, Tzyh-Chang Hwang.   

Abstract

Malfunction of cystic fibrosis transmembrane conductance regulator (CFTR), a member of the ABC protein superfamily that functions as an ATP-gated chloride channel, causes the lethal genetic disease, cystic fibrosis. This review focuses on the most recent findings on the gating mechanism of CFTR. Potential clinical relevance and implications to ABC transporter function are also discussed.

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Year:  2012        PMID： 23223629      PMCID： PMC4931552          DOI： 10.1152/physiol.00026.2012

Source DB:  PubMed          Journal:  Physiology (Bethesda)        ISSN： 1548-9221


  84 in total

1.  An inward-facing conformation of a putative metal-chelate-type ABC transporter.

Authors:  H W Pinkett; A T Lee; P Lum; K P Locher; D C Rees
Journal:  Science       Date:  2006-12-07       Impact factor: 47.728

2.  Thermodynamics of CFTR channel gating: a spreading conformational change initiates an irreversible gating cycle.

Authors:  László Csanády; Angus C Nairn; David C Gadsby
Journal:  J Gen Physiol       Date:  2006-10-16       Impact factor: 4.086

3.  The E. coli BtuCD structure: a framework for ABC transporter architecture and mechanism.

Authors:  Kaspar P Locher; Allen T Lee; Douglas C Rees
Journal:  Science       Date:  2002-05-10       Impact factor: 47.728

4.  Gating of cystic fibrosis transmembrane conductance regulator chloride channels by adenosine triphosphate hydrolysis. Quantitative analysis of a cyclic gating scheme.

Authors:  S Zeltwanger; F Wang; G T Wang; K D Gillis; T C Hwang
Journal:  J Gen Physiol       Date:  1999-04       Impact factor: 4.086

5.  Cystic fibrosis: CFTR correctors to the rescue.

Authors:  David N Sheppard
Journal:  Chem Biol       Date:  2011-02-25

Review 6.  ABC multidrug transporters: structure, function and role in chemoresistance.

Authors:  Frances J Sharom
Journal:  Pharmacogenomics       Date:  2008-01       Impact factor: 2.533

7.  Alternating access in maltose transporter mediated by rigid-body rotations.

Authors:  Dheeraj Khare; Michael L Oldham; Cedric Orelle; Amy L Davidson; Jue Chen
Journal:  Mol Cell       Date:  2009-02-27       Impact factor: 17.970

8.  Alignment of transmembrane regions in the cystic fibrosis transmembrane conductance regulator chloride channel pore.

Authors:  Wuyang Wang; Yassine El Hiani; Paul Linsdell
Journal:  J Gen Physiol       Date:  2011-07-11       Impact factor: 4.086

9.  CFTR gating I: Characterization of the ATP-dependent gating of a phosphorylation-independent CFTR channel (DeltaR-CFTR).

Authors:  Silvia G Bompadre; Tomohiko Ai; Jeong Han Cho; Xiaohui Wang; Yoshiro Sohma; Min Li; Tzyh-Chang Hwang
Journal:  J Gen Physiol       Date:  2005-03-14       Impact factor: 4.086

10.  The ClC-0 chloride channel is a 'broken' Cl-/H+ antiporter.

Authors:  Jirí Lísal; Merritt Maduke
Journal:  Nat Struct Mol Biol       Date:  2008-07-20       Impact factor: 15.369
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  20 in total

1.  Structural mechanisms for defective CFTR gating caused by the Q1412X mutation, a severe Class VI pathogenic mutation in cystic fibrosis.

Authors:  Jiunn-Tyng Yeh; Ying-Chun Yu; Tzyh-Chang Hwang
Journal:  J Physiol       Date:  2018-12-02       Impact factor: 5.182

2.  Synergistic Potentiation of Cystic Fibrosis Transmembrane Conductance Regulator Gating by Two Chemically Distinct Potentiators, Ivacaftor (VX-770) and 5-Nitro-2-(3-Phenylpropylamino) Benzoate.

Authors:  Wen-Ying Lin; Yoshiro Sohma; Tzyh-Chang Hwang
Journal:  Mol Pharmacol       Date:  2016-07-13       Impact factor: 4.436

Review 3.  Cystic fibrosis transmembrane conductance regulator chloride channel blockers: Pharmacological, biophysical and physiological relevance.

Authors:  Paul Linsdell
Journal:  World J Biol Chem       Date:  2014-02-26

4.  State-dependent blocker interactions with the CFTR chloride channel: implications for gating the pore.

Authors:  Paul Linsdell
Journal:  Pflugers Arch       Date:  2014-03-28       Impact factor: 3.657

Review 5.  Architecture and functional properties of the CFTR channel pore.

Authors:  Paul Linsdell
Journal:  Cell Mol Life Sci       Date:  2016-10-03       Impact factor: 9.261

6.  Cysteine scanning of CFTR's first transmembrane segment reveals its plausible roles in gating and permeation.

Authors:  Xiaolong Gao; Yonghong Bai; Tzyh-Chang Hwang
Journal:  Biophys J       Date:  2013-02-19       Impact factor: 4.033

Review 7.  Structural mechanisms of CFTR function and dysfunction.

Authors:  Tzyh-Chang Hwang; Jiunn-Tyng Yeh; Jingyao Zhang; Ying-Chun Yu; Han-I Yeh; Samantha Destefano
Journal:  J Gen Physiol       Date:  2018-03-26       Impact factor: 4.086

Review 8.  CFTR potentiators: from bench to bedside.

Authors:  Kang-Yang Jih; Wen-Ying Lin; Yoshiro Sohma; Tzyh-Chang Hwang
Journal:  Curr Opin Pharmacol       Date:  2017-11-05       Impact factor: 5.547

9.  Full-open and closed CFTR channels, with lateral tunnels from the cytoplasm and an alternative position of the F508 region, as revealed by molecular dynamics.

Authors:  Jean-Paul Mornon; Brice Hoffmann; Slavica Jonic; Pierre Lehn; Isabelle Callebaut
Journal:  Cell Mol Life Sci       Date:  2014-10-07       Impact factor: 9.261

10.  On the mechanism of gating defects caused by the R117H mutation in cystic fibrosis transmembrane conductance regulator.

Authors:  Ying-Chun Yu; Yoshiro Sohma; Tzyh-Chang Hwang
Journal:  J Physiol       Date:  2016-03-23       Impact factor: 5.182
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